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Paget's Disease of Bone

Terms in this set (29)

What is another name for Paget's Disease of Bone?
Osteitis Deformans
What is the problem in Paget's Disease of bone?
Imbalance between osteoclasts and osteoblasts
Paget's Disease of Bone Epidemiology:
What population does this affect?
Arises in late adulthood (over 50)
Paget's Disease of Bone Epidemiology:
This arises in late adulthood (over 50). What is the cause of this?
Cause is not clear
--Possibly some viral infection causes the osteoclasts to go crazy
Paget's Disease of Bone Epidemiology:
This is an imbalance between osteoclasts and osteoblasts. Is this systemic or local?
Local
--Only affects one or a few bones
Paget's Disease of Bone Epidemiology:
This is a local process that only affects one or a few bones. What bones are often involves?
Pelvis, skull, femur
Paget's Disease of Bone Pathogenesis:
What are the three phases of disease?
Early = osteoclastic phase
Middle = mixed osteoblast-osteoclast phase
Late = osteoblastic phase
Paget's Disease of Bone Pathogenesis:
What is occurring during the early stages of disease?
Osteoclastic phase
--Osteoclasts are going crazy and resorbing bone
Paget's Disease of Bone Pathogenesis:
What is occurring during the middle stages of disease?
Mixed osteoblast-osteoclast phase
--Osteoblasts respond to the increased bone resorption by increasing bone formation
Paget's Disease of Bone Pathogenesis:
What is occurring during the late stages of disease?
Osteoblastic phase
--Eventually, osteoclasts burn out and osteoblasts are left unopposed
Paget's Disease of Bone Pathogenesis:
What is the end result after the late stages of disease?
Osteoblasts are unopposed
--Thick, sclerotic bone that easily fractures
Paget's Disease of Bone Pathogenesis:
The end result is thick, sclerotic bone that easily fractures. What would be seen on a biopsy of the bone?
Mosaic pattern of lamellar bone
Paget's Disease of Bone Pathogenesis:
A mosaic pattern of lamellar bone will be seen on biopsy. What does this mean?
Bone looks like 'puzzle pieces'
--Bone is being made, but not being fused together
Paget's Disease of Bone Clinical Features:
There is an abnormal process going on in the bone. How will this present?
Bone pain
Paget's Disease of Bone Clinical Features:
At the end of the disease, there is unopposed osteoblast action. How can this affect hat size and why?
Increasing hat size
--Due to osteoblast activity that enlarges the skull
Paget's Disease of Bone Clinical Features:
How can hearing be affected?
Thickening of the skull can impinge on cranial nerves
--CN VIII can be compressed, causing hearing loss
Paget's Disease of Bone Clinical Features:
What characteristic facial changes can be seen?
'Lion-like' facies
--Due to tons of bone being made in the craniofacial bones
Paget's Disease of Bone Lab Findings:
What lab findings would be found?
-Calcium?
-Phosphate?
-PTH?
-Alkaline phosphatase?
Calcium, phosphate, and PTH all normal
Elevated alk phos
Paget's Disease of Bone Lab Findings:
What lab finding is used to diagnose Paget's?
Isolated elevated alkaline phosphatase
--Paget's = MCC of isolated elevated alk phos in pts over 40
Paget's Disease of Bone Lab Findings:
This is diagnosed by an isolated elevated alk phos. Why does this occur?
Overactive osteoblasts make alk phos
Paget's Disease of Bone:
What would be seen on x-ray?
Thickened bone with shaggy areas of radiolucency
Paget's Disease of Bone Complications:
The bone is not being formed properly. What complication can this lead to?
Can lead to pathologic fractures
--Bone is weak and fractures easily
Paget's Disease of Bone Complications:
While the bone is remodeling, the capillaries can be messed up as well. What can this lead to?
High-output cardiac failure
Paget's Disease of Bone Complications:
How can this lead to high-output cardiac failure?
During the remodeling process, the capillaries can be messed up too
--AV shunts develop

Blood isn't getting into the capillaries
Paget's Disease of Bone Complications:
What tumor does this predispose to?
Risk for osteosarcoma
Paget's Disease of Bone Complications:
There is an increased risk for osteosarcoma. Why?
Late disease, the osteoblasts are overworking
--Easy to become a little more malignant
Paget's Disease of Bone Treatment:
What endogenous hormone can be used to inhibit the osteoclast function?
Calcitonin
Paget's Disease of Bone Treatment:
What drugs can be used to stabilize the bone and inhibit osteoclast function?
Bisphosphonates
--Stabilizes hydroxyapatite
--Inhibits osteoclasts and promotes apoptosis
Paget's Disease of Bone Treatment:
Calcitonin and bisphosphonates can be used to limit osteoclast function. What stage of disease are they useful for?
Useful during early stages (osteoclastic phase)
--Not useful late in disease
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